AB1419 IS DECREASED BODY MASS INDEX -2 Z SCORE OR LESS CORRELATING WITH AN ORGAN INVOLVEMENT PATTERN? RESULTS FROM THE JUVENILE SCLERODERMA INCEPTION COHORT
Authors: I. Foeldvari, J. Klotsche, K. Torok, O. Kasapcopur, A. Adrovic, B. Feldman, F. R. Sztajnbok, J. Antón, M. T. Terreri, A. P. Sakamoto, R. Khubchandani, V. Stanevicha, E. Al-Abadi, E. Alexeeva, M. Katsikas, S. Sawhney, D. Schonenberg, V. Smith, S. Appenzeller, T. Avcin, T. Lehman, H. Malcova, E. Marrani, A. Patwardhan, W. A. Sifuentes-Giraldo, N. Vasquez-Canizares, P. Costa Reis, M. Janarthanan, S. Johnson, M. Kostik, M. Moll, D. Nemcova, C. Pain, M. J. Santos, S. Abu Al Saoud, C. Battagliotti, L. Berntson, B. Bica, J. Brunner, D. Eleftheriou, L. Harel, G. Horneff, D. Kaiser, T. Kallinich, D. Lazarevic, K. Minden, F. Nuruzzaman, S. Opsahl Hetlevik, Y. Uziel, N. Helmus
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. In adult patients decreased body mass index (BMI) correlates with higher mortality. We hypothesized that jSSc patients with lower BMI at presentation have more severe organ involvement. <h3>Objectives</h3> To compare the organ involvement pattern at inclusion into the cohort of jSSc patients with BMI ≤ -2 z score with the patients with higher BMI. <h3>Methods</h3> We reviewed the clinical characteristics of patients who were recruited to the juvenile jSScC till 1<sup>st</sup> of December 2022. We compared patients with BMI ≤ -2 z score with patients (lwgroup) with higher BMI (nlwgroup). jSScC is a prospective cohort of jSSc patients, who developed the first non-Raynaud´s symptom before the age of 16 years and are under the age of 18 years at the time of inclusion. <h3>Results</h3> At the time of the evaluation, we had 232 patients in the cohort and 217 of them had BMI data to include in the evaluation. Thirty-three patients were in the lwgroup (15%) and 88% (n=29/33) of them diffuse subtype and in the nlwgroup 64% (113/177). The median age at onset of Raynaud phenomenon in the whole group was 10.6 years and the median age at the first non-Raynaud symptom in the whole group was 11.0 years. Median disease duration in the whole group was 2.4 years at the time of inclusion. Approximately 95% of the patients were treated with a DMARD. There were no statistically significant differences between the lwgroup compared to nlwgroup regarding antibody pattern, inflammatory marker or organ involvement pattern, except higher number of patients with Gottron papules (41% lwgroup vs 25% nlwgroup; p=0.01) and sclerodactylia (84% lwgroup vs 73 % nlwgroup; p=0.049). Regarding the patient related outcomes at inclusion in the cohort, the global disease activity by VAS 0-100 was 40 in both groups (p=0.032), but the patient global disease damage by VAS 0-100 was 50 in the lwgroup which was significantly higher compared to 30 nlwgroup (p=0.014). <h3>Conclusion</h3> In our jSSc cohort, currently the largest of the world, we could not find any differences regarding major internal organ involvement in patients with lower BMI at time of inclusion in the cohort. Nevertheless, there is a significant difference in patient related outcomes regarding global organ damage between the two groups. The long-term prognosis of these patients should be addressed in future studies. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.